Hydrocephalus: The Past, the Present, and the Future

Hydrocephalus is a neurological disorder where there is a fluid buildup in the brain. Diagnosis and treatment of hydrocephalus has improved dramatically. This disease can happen to anyone; it is not a genetic disorder, and it can be diagnosed at anytime. Physically, mobility is often a difficulty. The fluid buildup leads to constant headaches, anorexia, lethargy, and a plethora of other health concerns. The resolution to these problems is a shunt. Emotionally, acceptance can be hard for those diagnosed later in life. Depression is also common for some; medications are prescribed to help.

The history of hydrocephalus leads all the way back to 1550 when researchers first introduced noncommunicating hydrocephalus ("Timeline Results for Hydrocephalus History." Web. 19. Apr. 2010). Chronic hydrocephalus was introduced in 1593(Sauvages, The Cyclopedia of Practical Medicine. Google.org). This was also the year that researchers put the abnormal shape of babies’ head and the disease together. In 1891, a spinal tap was used to treat the disease (Aschoff, Kremer, Hashemi, and Kunze, "The Scientific History of Hydrocephalus and its Treatment. Neurosurgical Review). In the 1950’s, John Holter, a researcher, came up with the first effective shunt for his son. A decade later, the shunt procedure was explained. Today the process for treating hydrocephalus is much that same as it was in the 1950’s. However, there are now many different types of shunts.

Centuries ago, children with hydrocephalus weren’t treated. In fact, children with hydrocephalus were seen as aliens or freaks (Najaflan, Alireza. "Severe Hydrocephalus." Goldbaboo.jpg). Before the 1950’s, hydrocephalus was usually fatal. When shunts started to be used, the prognosis was 46% fatality (Fried, A. "Childhood Hydrocephalus: Clinical Features, Treatment and Slit-Ventricle Syndrome." virtualtrials.com). Today, many people with hydrocephalus are successfully treated, although they may have a variety of other issues.

A child may develop hydrocephalus before they are born. This can be detected by the use of an ultrasound. Usually the doctor has to take the baby by caesarian section. These babies generally have a lot more problems because there has been a buildup of cerebral spinal fluid (CSF) ("Symptoms and Diagnostic Tests." hydroassoc.org).

Babies may also develop hydrocephalus when they are born. The two common causes of this are a bleed in the brain or an infection that goes into the brain. Babies that develop hydrocephalus often develop a large head, the soft spot becomes hard and bulges, the veins in the head stand out, and the eyes drop down and you can’t see them much. Also most babies with hydrocephalus become irritable, lethargic, don’t eat, and tend to throw up a lot ("Hydrocephalus." neurosurgerytoday.org. What is Neurosurgery?).

In toddlers and children the symptoms may be the same as in babies. However, additional symptoms may include headaches, fever, personality changes, and trouble falling asleep or not being able to wake up. Physically, they become unstable in their balance, they have delayed progress in walking and talking, poor coordination, and lose much of the ability to do things they were previously able to do ("Hydrocephalus." Neurosurgerytoday.org. What is Neurosurgery?).

Young and middle aged adults who develop hydrocephalus usually have headaches, problems waking up or staying awake, poor coordination or balance, problems controlling their bladder, and impaired vision and cognitive skills. For older adults the symptoms vary a bit. Their coordination or balance becomes poor to the point where they shuffle their feet when they walk. They also have headaches, memory loss, and problems controlling their bladder("Hydrocephalus." Neurosurgerytoday.org What is Neurosurgery?).

There are many different causes of hydrocephalus. For babies and children congenital malformations are a major cause of hydrocephalus(Fried, A. "Childhood Hydrocephalus: Clinical Features, Treatment, and the Slit-Ventricle Syndrome" virtualtrials.com). These malformations don’t allow the CSF to drain from the brain. Instead the CSF stays in the brain, pressing against the brain cells. In a normal brain, 80% of the brain consists of brain cells, 10% is blood, and 10% is CSF. If more than 10% of the brain is made up of CSF, the percentage of brain cells decreases. There are three other common causes of hydrocephalus in babies and children. One is an intraventricular hemorrhage in the brain, another is a tumor, and the third is an injury to the brain ("Hydrocephalus Fact Sheet." National Institute of Neurological Disorders and Strokes. ninds.nih.gov). The most common cause of hydrocephalus in teenagers through middle aged adults is head trauma. For elderly people, hydrocephalus is associated with Alzheimer’s and Parkinson’s diseases.

At first, doctors will diagnose hydrocephalus by monitoring the size of the head growth (in infants), taking a detailed history, and using a CT scan to see if the ventricles are enlarged. Sometimes an MRI may be used as a diagnostic tool. In rare cases doctors may measure the intracranial pressure (ICP) by placing a monitor in the brain to determine the pressure in the brain or by doing a lumbar puncture. Normal ICP is between 1 and 18 mm of mercury("Intracranial Pressure Monitoring. US National Library of Medicine and National Institute of Health. ninds.nih.gov).

The normal course of treatment for hydrocephalus is placing a shunt into the patient. The shunt drains the extra CSF off the brain or spine. Usually, it drains into the abdominal cavity (ventricular peritoneal shunt). However there are other places the CSF can be drained to. This includes the heart (ventricular atrial shunt), lungs (ventricular pleural shunt), ureter, or gallbladder. If they find that a baby has hydrocephalus before being born, a doctor will put a shunt in when the baby is 2 days old ("Hydrocephalus." wikipedia.org).

To place a shunt in a patient, a surgical cut is made through the scalp, behind the ear. There is another incision made in the abdomen (or wherever they place the end of the shunt). The doctor drills a small hole in the skull. There is a small tube that is put through the opening, through the brain, and into the ventricle. A valve is connected to the shunt and placed on the outside of the skull. Another tube is placed in the valve and taken under the skin to the location of the drain. The incisions are closed up and the patient goes to recovery.

There are several different types of shunts ("Cerebral Shunt." wikipedia.org). There is a Delta valve that helps prevent the shunt from taking too much CSF off. If too much CSF is siphoned off, you will end up with severe headaches and you have to stay down. Another shunt is a Medium Pressure Cylindrical valve which may lead to uneven drainage from the ventricles. This would cause you to fluctuate from feeling good to awful (actually horrible).

There is also the Nulsen and Spitz valve. This was the first mass produced valve that they used to treat hydrocephalus (Aschoff, Kremer, Hashemi, and Kunze, "The Scientific History of Hydrocephalus and Its Treatment. Neurosurgical Review). This contains two ball-valve units connected with a spring. It does not have an adjustable pressure setting. Without the adjustable pressure setting more CSF is likely to drain off when you stand or sit up. An Anti-Siphon device can be put in to help so the shunt doesn't take too much CSF off (Fried, A. "Childhood Hydrocephalus:Clinical Features, Treatment, and Slit-Ventricle Syndrome." virtuals.com).

The Sigma valve operates on a flow-control mechanism. This is completely different than how the other valves work, which are pressure-controlled systems. This Sigma valve can be adjusted to change the amount of CSF being drained by use of a magnetic device. This means you wouldn’t need surgery to change the pressure ("Cerebral Shunt" wikipedia.org).

Shunts seen like an easy solution, but they aren’t. There are risks when placing a shunt in a patient. There can be bleeding in the brain or brain swelling. The shunt could get infected. There may be an infection in the brain or damage to the brain tissue. The person may also have seizures. If the shunt is successfully placed, too much CSF can be drained. The most common problem with a shunt is that it can quit working. This means another surgery. Currently, in the United States, there is one shunt surgery every 13 minutes, with a cost of over 1 billion dollars. After having a shunt inserted, there is a 50% chance that a revision will be needed within 2 years ("Cerebral Shunt" wikipedia.org). For some people it can be far more than that.

Even if the hydrocephalus is treated, most individuals have some type of cognitive and/or physical impairment. This is why it is so important to find ways to prevent and better treat hydrocephalus. Hydrocephalus, especially which related to brain injuries, may be preventable. People should always wear a helmet when riding their bikes, using roller blades or when skiing. Also helmets should be worn by motorcyclists. People should always wear a helmet when riding their bikes, using roller blades, or when skiing. Also helmets should be worn by motorcyclists. People in cars should always wear a seat belt to prevent their heads from being bumped. Children and adults should wear helmets with any contact sports. Stop and think. Anytime your head can get bumped, it’s best to protect it.

You or someone you know could become hydrocephalic. The Hydrocephalus Association has a wonderful mission. Their mission is, “To eliminate the challenges of hydrocephalus by stimulating innovative research and providing support, education, and advocacy for individuals, families and professionals dealing with hydrocephalus.” Please join this non-profit organization that works so hard to make the lives of people with hydrocephalus better. Their website is: http://www.hydroassoc.org/.